[Chyluria caused by congenital retroperitoneal lymphangiectasis]. / Chylurie door congenitale retroperitoneale lymfangiëctasieën.

A Dutch woman with non-parasitic chyluria is described. Lymphangiography and abdominal CT demonstrated retroperitoneal lymphangiectasis, dilated and tortuous lymphatic ducts around the right pyelocaliceal system and contrast in the bladder. The thoracic duct was patient. There were no metabolic or immunological abnormalities. The aetiology was probably congenital malformation of the lymphatic system. The treatment was conservative.

Grades of nodular sclerosis (NSI-NSII) in Hodgkin's disease. Are they of independent prognostic value?

Subclassification of the nodular sclerosis (NS) type of Hodgkin's disease in Grade 1 and 2 was reported for the first time by the British National Lymphoma Investigation (BNLI). Three groups, the BNLI, Gärtner et al. and the current authors, found clearly different survival rates between Grade 1 and 2 NS patients. The authors studied retrospectively if this NS grading has an independent prognostic value in 90 NS patients, diagnosed in ten hospitals in the southeastern part of the Netherlands (1972-1983). In this study there is no significant difference in sex, age, B-symptoms, erythrocyte sedimentation rate (ESR), stage, positive laparotomy, involvement of mediastinum or spleen, lymphocyte count, and percentage of complete remissions between the NS subgroups. Multivariate analysis suggests that the subclassification of NS in Grades 1 and 2 is a prognostic factor in survival independent of age, stage and ESR. This finding and the high relative frequency of NS makes application of this NS subdivision probably clinically useful to identify patients for a risk-adapted therapy.

Phase II trial of 5-fluorouracil, adriamycin and cisplatin (FAP) in advanced gastric cancer.

Twenty patients (15 male, 5 female) with nonresectable gastric adenocarcinoma were treated with FAP (5-fluorouracil 300 mg/m2 IV on days 1-5, adriamycin 50 mg/m2 IV on day 1, cisplatin 20 mg/m2 IV on days 1-5). Each course was repeated every 21 days. Eighteen patients were evaluable for response. The median age was 51 years, the range extending from 34 to 68. None had undergone chemotherapy. The median Karnofsky performance score was 80%. Nine (50%) partial responses (PR) and eight (44%) cases of stable disease (SD) were observed. One patient showed progression of the disease and died after 6 months. The median duration of response was 6+ months for PR and 6 months for SD. The median survival was 12 months. FAP toxicity was moderate, with the median WBC nadir 3.2 X 10(9)/l (range 0.7-4.2). One patient in PR died of septicemia. Nausea and vomiting were not dose-limiting. Neuropathy was mild in four and moderate in two patients. This FAP combination appears to be as effective with respect to response rate and duration as reported for 5-fluorouracil, adriamycin and mitomycin C (FAM).

Successful treatment by plasmapheresis of respiratory insufficiency in myasthenia gravis.

A 27-year-old man with myasthenia gravis developed a severe myasthenic crisis with respiratory insufficiency. In spite of high iv doses of pyridostigminebromide and corticosteroids, mechanical ventilation remained necessary. Treatment with plasma exchange grossly decreased the serum level of antibodies against acetylcholine receptor protein and caused an immediate clinical improvement. The respiratory assistance could be terminated and muscle strength increased substantially. Plasmapheresis seemed to be of definite benefit in the treatment of this acute crisis of myasthenia gravis.